CHARM ARROYO, RN

First defined in Japan by Dr. Tomikasu Kawasaki in 1967. It is a multisystem vasculitis identified by a febrile illness with several distinguishing features that attacks vital body system largely seen in children under 5 years of age. Also called Mucocutaneous lymph node syndrome, Infantile Polyarteritis.

Stage I Acute Febrile Phase (First 10 days) The appears severely ill and irritable Major diagnostic criteria established by CDC (Center for Disease Control and Prevention) are as follows: a. High, Spiking fever for 5 days or more b. Bilateral conjunctival injection c. Oropharyngeal erythema Strawberry tongue d. Erythema of hands and feet, periungal desquamation e. Erythematous generalized rash. f. Cervical lymphadenopathy greather than 0.6 inch (1.5cm) Pericarditis, myocarditis, cardiomegaly, heart failure, and pleural effusion. Other associated findings include meningitis, arthritis, sterile pyuria, vomiting, and diarrhea.

BILATERAL CONJUNCTINVAL INJECTION

STRAWBERRY TONGUE

ERYTHEMATOUS PALMS

ERYTHEMATOU S RASH ERYTHEMATOUS SOLES DESQUAMATION OF THE FINGERS ERYTHEMA AT THE SITE OF BCG VACCINE PERIANAL ERYTHEMATOUS DESQUAMATION

Stage II Sub acute Phase (Days 11 to 25) - Acute symptoms of stage I subside as temperature returns normal. The child remains irritable and anorexic. - Dry, cracked lips with fissures - Desquamation of toes and fingers. - Coronary thrombus, aneurysm, myocardial infarction, and heart failure. - Thrombocytosis peaks at 2 weeks.

Stage III Convalescent Phase (Until sedimentation rate and platelet count normalize) - The child appears well - Transverse grooves of fingers and toenails (Beaus lines). - Coronary thrombosis, aneurysms may occur.

The causative agent of Kawasaki disease is still unknown, but current theories center primarily on immunological causes for the disease. An association has been identified with an SNP in the ITPKC gene, which codes an enzyme that negatively regulates T-cell activation. An additional factor that suggests genetic susceptibility is the fact that regardless of where they are living, Japanese children are more likely than other children to contract the disease. The HLA-B51 serotype has been found to be associated with endemic instances of the disease.

Vasculitis Coronary Artery Aneurysm Myocardial Infarction Dysrrhythmia Aspirin toxicity

The diagnostic of Kawasaki disease is based on clinical manifestations. The CDC requires that fever and four of the six other criteria listed above in stage I be demonstrated. - Electrocardiogram, echocardiogram, cardiac catheterization, and angiocardiography may be required to diagnose cardiac abnormalities. - Although there are no specific laboratory tests, the following may help support diagnosis or rule out other disease.
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1. CBC leukocytosis during acute stage. 2. Erythrocytes and hemoglobin slight decrease. 3. Platelet count increased during second to fourth week of illness. 4. IgM, IgA, IgG, and IgF transiently elevated. 5. Urine protein and leukocytes present. 6. Acute phase reactants (ESR, C-reactive protein, alpha I antitrypsin) are elevated during the acute phase. 7. Myocardial enzyme levels (serum CK-MB) suggest MI if elevated. 8. Liver enzymes (AST, ALT) moderately elevated. 9. Lipid profile low high density lipoprotein and high triglyceride level.

1. Immune globulin (gamma globulin) I.V. therapy

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IVGG (2g/kg/day) is initiated during stage I in one 8 to 10 hour infusion to reduce the incidence of coronary artery abnormalities.

2. Aspirin therapy 3. Thrombolytic therapy

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may be required during stages I, II, or III.

4. Acetaminophen, Paracetamol

Coronary Artery Bypass Grafting (CABG) Heart Transplantation Stent Placement Cardiac Catheterization and Angiography Percutaneous Transluminal Coronary Rotational Angioplasty

Monitoring 1. Monitor pain level and childs response to analgesics. 2. Institute continual cardiac monitoring and assessment for complications; report arrhythmias. - Vital signs as directed by condition; report abnormalities. - Assess for signs of myocarditis (tachycardia, gallop rhythm, chest pain). -Monitor for heart failure (dyspnea, nasal flaring, grunting, retractions, cyanosis, orthopnea, crackles, moist respirations, distended jugular veins, edema). 3. Closely monitor intake and output, and administer oral and I.V fluids as ordered. 4. Monitor hydration status by checking skin turgor, weight, urinary output, specific gravity, and presence of tears. 5. Observe mouth and skin frequently for signs of infection.

Supportive Care

the child periods of uninterrupted rest. Offer pain medication routinely rather than as needed during stage I. Avoid NSAIDS if the child is in aspirin therapy. 2. Perform comfort measures related to the eyes. - Conjunctivitis can cause photosensitivity, so darken the room, offer sunglasses. - Apply cool compress. - Discourage rubbing the eyes. - Instill artificial tears to soothe conjunctiva. 3. Monitor temperature every 4 hours. Provide sponge bath if temperature above normal. 4. Perform passive range of motion exercises every 4 hours while the child is awake because movement may be restricted. 5. Provide quiet and peaceful environment with diversional activities.

1. Allow

6. Provide care measures for oral mucous membrane. - Offer cool liquids like ice chips and ice pops. - Use soft toothbrush only. - Apply petroleum jelly to dried, cracked lips. 7. Provide skin measures to improve skin integrity. - Avoid use of soap because it tends to dry skin and make it more likely to breakdown. - Elevate edematous extremities. - Use smooth sheets. - Apply emollients to skin as ordered. - Protect peeling of skin, observe for signs of infection. 8. Offer clear liquids every hour when the child is awake. 9. Encourage the child to eat meals and snack with adequate protein. 10. Infuse I.V fluids through a volume control device if dehydration is present, and check the site and amount hourly. 11. Explain all procedures to the child and family.

11. Explain all procedures to the child and family. 12. Encourage the parents and child to verbalize their concerns, fears, and questions. 13. Practice relaxation techniques with child, such as relaxation breathing, guided imagery, and distraction. 14. Prepare the child for cardiac surgery or thrombolytic therapy if complications develop. 15. Keep the family informed about progress and reinforce stages and prognosis.

With early treatment, rapid recovery from the acute symptoms can be expected and the risk of coronary artery aneurysms greatly reduced. If left untreated, the acute symptoms of Kawasaki disease are self- limited but the risk of coronary artery involvement is much greater. Overall, about 2% of patients die from complications of coronary vasculitis. Patients who have had Kawasaki disease should have an ECG initially every few weeks, and then every one- two years to screen for progression of cardiac involvement.